Hairy-cell leukemia

Hairy cell Leukemia (HCL) is a chronic lymphoproliferative disorder that is defined, according to the WHO classification, as a mature (peripheral) B-cell neoplasm. HCL accounts for between 2-3% of all leukemia cases, with about 600 new cases diagnosed in the U.S. each year. With regard to genetic abnormalities of potential pathogenetic importance in HCL, only a few have been described. Mutations of p53 and Bcl-6 have been reported in about one-third of cases, but their functional significance is unclear. Over-expression of cyclin D1, an important cell cycle regulator, has been reported in cases of HCL as well as mantle cell lymphoma (MCL) and in a few cases of chronic lymphocytic leukemia among lymphoid malignancies.

Cancer

p53 (mutation) [HSA:7157] [KO:K04451]
Bcl-6 (mutation) [HSA:604] [KO:K15618]
Cyclin D1 (overexpression) [HSA:595] [KO:K04503]

CD103 [HSA:3682]
CD11c [HSA:3687]
CD25 [HSA:3559]
Annexin A1 [HSA:301]

Cladribine (2-chlorodeoxyadeosine; CdA) [DR:D01370]

ICD-O: 9940/3, Tumor type: Hairy cell leukemia

PMID:12670461 (gene, tumor type)

Zuzel M, Cawley JC.

The biology of hairy cells.

Best Pract Res Clin Haematol 16:1-13 (2003)

PMID:16880237 (marker)

Wanko SO, de Castro C.

Hairy cell leukemia: an elusive but treatable disease.

Oncologist 11:780-9 (2006)

PMID:15599661 (marker)

Allsup DJ, Cawley JC.

Diagnosis, biology and treatment of hairy-cell leukaemia.

Clin Exp Med 4:132-8 (2004)

PMID:16723990 (marker)

Tiacci E, Liso A, Piris M, Falini B.

Evolving concepts in the pathogenesis of hairy-cell leukaemia.

Nat Rev Cancer 6:437-48 (2006)

(drug)

McPhee SJ, Papadakis MA, Tierney LM (ed).

Current Medical Diagnosis & Treatment 2007, Forty-Sixth Edition

The McGraw-Hill Companies, Inc. (2007)